Malignant Perivascular Epithelioid Cell Tumor (PEComa) of the Uterus

This is a case of a 52-year-old woman who underwent a hysterectomy to treat a large uterine perivascular epithelioid cell tumor. She was followed with serial F-18 FDG PET/CT scans due to positive surgical margins and vascular invasion on pathology. Initial surveillance PET/CT performed 6 months post surgery was negative; however, a second surveillance PET/CT performed 12 months post surgery showed an intensely FDG-avid recurrence in the pelvic surgical site. The patient received local radiotherapy, 2 cycles of taxol and carboplatin, and was placed on imatinib mesylate (Gleevec). A final PET/CT performed 6 months after initiation of therapy showed rapidly disseminating metastatic disease and the patient died 1 month later. This rare report highlights a potentially new utility of F-18 FDG PET/CT for surveillance of recurrence of a malignant uterine perivascular epithelioid cell tumor, as well as for evaluation of response to therapy.